Evolution of diagnosis and treatment for lupus nephritis in Spain.

Lupus nephritis (LN) is a serious manifestation of systemic lupus erythematosus that can lead to end-stage renal disease. Many clinical and prognostic data on which our therapeutic decisions are based come from international cohorts, which have important ethnic and prognostic differences. To identify clinical and prognostic data from patients with LN in Spain, we undertook a bibliographic search of NL-related papers by Spanish authors and published in national and international journals between 2005 and 2022. According to the selected references, renal biopsy is not only essential for LN diagnosis but its repetition can be useful for the follow-up. Regarding LN treatment, standard strategy consists of an induction phase and a maintenance phase. However, as new drugs have been released, a new paradigm of treatment in a single, continuing and personalized phase has been proposed.

Persistent Facial Oedema and Erythema in a Woman, An Uncommon Manifestation of Chronic Cutaneous Lupus Erythematosus.

Manifestations of chronic cutaneous lupus erythematosus are variable. Periorbital and facial swelling occurs in dermatomyositis and systemic lupus, but it has been rarely reported as a manifestation of exclusively cutaneous lupus. A 48-year-old woman presented with a 16-year history of asymptomatic, bilateral swelling and erythema of her face with marked worsening after sun exposure. No systemic symptoms were associated. A complete evaluation did not reveal other findings. Cutaneous biopsy showed features of lupus erythematosus. She was treated with photoprotection, topical tacrolimus, hydroxychloroquine and azathioprine with a partial response. Facial swelling with erythema represents quite an unusual manifestation of chronic cutaneous lupus erythematosus. Dermatomyositis, systemic lupus and Morbihan disease are the main differential diagnoses. LEARNING POINTS: Periorbital and facial swelling with erythema are clinical manifestations of dermatomyositis and systemic lupus erythematosus. However, these manifestations represent quite an unusual presentation of chronic cutaneous lupus erythematosus.The periorbital area is most frequently affected, while extensive facial involvement is much more unusual.A complete evaluation and cutaneous biopsy are essential to make the diagnosis and to rule out other disorders such as dermatomyositis, systemic lupus erythematosus and Morbihan disease.

Implementation of positron emission tomography with 18F-fluorodeoxyglucose associated with computed tomography in patients with sarcoidosis. Its effect on disease staging and the therapeutic approach

Objetives: to evaluate the benefit of implementing 18F-FDG PET/TC in the staging and treatment adjustment of patients with sarcoidosis, compared with the signs and symptoms and complementary test results usually employed. Materials and methods: an observational, analytical electronic chart review of a retrospective cohort of patients seen for sarcoidosis in the internal medicine department of a Spanish university hospital. Results: a total of 31 patients (18 males) were evaluated, with an average age of 54.6±14.71 years and 11±5.75 years since their sarcoidosis diagnosis. In the 84.6% of the reviews, positive uptake was objectified on the 18F-FDG PET/TC. In the 42.3% of the occasions, the objectified find ing allowed restaging of the patient. The 18F-FDG PET/TC result justified the choice of treatment in the 71% of the reviews. Conclusions: 18F-FDG PET/TC provided additional advantages in the staging and therapeutic management of patients with sarcoidosis, compared with the evaluation of signs and symptoms and other clinical tests usually employed in follow up, due to its greater accuracy in determining the activity and extension of the disease. (Acta Med Colomb 2022; 48. DOI: https://doi.org/10.36104/amc.2023.2778).

[Kikuchi disease: Evolution during outbreaks and its response to antimalarial treatment]. / Enfermedad de Kikuchi-Fujimoto: evolución en brotes con respuesta a antipalúdicos.

Kikuchi-Fujimoto disease, also known as histiocytic necrotizing lymphadenitis, is a syndrome characterized by the presence of usually painful cervical lymphadenopathy with fever and night sweats. It is a rare clinical entity that mainly affects young Asian women, although it found worldwide. It is a benign and self-limiting condition; however, its importance lies in its differential diagnosis with other clinical entities such as lymphoma or histiocytoma. We present a case of Kikuchi-Fujimoto disease, with special emphasis on its clinicopathological significance and its evolution during outbreaks. Antimalarial therapy causes remission and we discuss the use of hydroxychloroquine for reoccurring cases.

Evolución del diagnóstico y tratamiento de la nefritis lúpica en España / Evolution of diagnosis and treatment for lupus nephritis in Spain

La nefritis lúpica (NL) es una manifestación grave del lupus eritematoso sistémico que puede llevar a una enfermedad renal terminal. La mayor parte de los datos clínicos y pronósticos que manejamos, y sobre los que tomamos decisiones terapéuticas, proceden de cohortes internacionales con importantes diferencias étnicas y relativas al pronóstico renal. Para conocer los datos clínicos y pronósticos de los pacientes con NL en España se realizó una búsqueda bibliográfica de artículos relacionados con la NL publicados por autores españoles en revistas nacionales e internacionales entre 2005 y 2022. Las referencias seleccionadas mostraron que la biopsia no solo es clave en el diagnóstico de la NL, sino que su repetición puede ser útil en el seguimiento. En cuanto al tratamiento el abordaje estándar de la NL consiste en una fase de inducción y una fase de mantenimiento. Sin embargo, la aparición de nuevos fármacos ha motivado que se postule un nuevo paradigma de tratamiento en una sola fase continuada y personalizada (AU)

Lupus nephritis (LN) is a serious manifestation of systemic lupus erythematosus that can lead to end-stage renal disease. Many clinical and prognostic data on which our therapeutic decisions are based come from international cohorts, which have important ethnic and prognostic differences. To identify clinical and prognostic data from patients with LN in Spain, we undertook a bibliographic search of LN-related papers by Spanish authors and published in national and international journals between 2005 and 2022. According to the selected references, renal biopsy is not only essential for LN diagnosis but its repetition can be useful for the follow-up. Regarding LN treatment, standard strategy consists of an induction phase and a maintenance phase. However, as new drugs have been released, a new paradigm of treatment in a single, continuing and personalized phase has been proposed (AU)

Pioderma gangrenoso, complicación infrecuente en la neuroestimulación medular / Pyoderma gangrenosum, undocumented complication of a spinal cord stimulation

El pioderma gangrenoso (PG) es una enfermedad cutánea rara y dolorosa con una respuesta de tratamiento impredecible. Las lesiones son recurrentes y ocurren en áreas que han sufrido traumas previos. Algunos casos se han relacionado con diferentes tipos de cirugías y, por lo general, no se diagnostican correctamente y se clasifican erróneamente como un proceso infeccioso. La implantación de un sistema de neuroestimulación es un procedimiento quirúrgico con varias complicaciones posibles, generalmente infecciosas, pero también no infecciosas, como reacciones de hipersensibilidad, pero no hay una descripción de la PG. Presentamos un paciente con un síndrome de dolor regional complejo posquirúrgico en ambos pies que desarrolla una lesión necrótica, pustulosa, eritematosa y dolorosa después del implante de un sistema de neuroestimulación medular que se diagnosticó como PG y se trató correctamente gracias a un enfoque multidisciplinario

Pyoderma gangrenosum (PG) is a rare and painful skin disease with unpredictable treatment response. The lesions are recurrent and occur in areas that have suffered previous trauma. Some cases have been related to different types of surgeries and it is usually underdiagnosed and erroneously classifi ed as an infectious process. The implantation of a neurostimulation system is a surgical procedure with several possible complications, usually infectious but also non-infectious such as hypersensitivity reactions but there is no description of PG. We report a patient with a post-surgical complex regional pain syndrome in both feet that develops a necrotic, pustular, erythematous and painful lesion after the implantation of a spinal cord stimulation which was diagnosed as PG and correctly treated thanks to a multidisciplinary approach

Cryoglobulinemia with acronecrosis not associated with hepatitis C infection: a case report.

Cryoglobulinemia is a rare disease characterized by the production of monoclonal or polyclonal immunoglobulins that precipitate in cold temperature. While this phenomenon can be observed in a large number of disorders, it has been associated with hepatitis C virus infection in more than 90% of cases. The remaining 10%, called essential cryoglobulinemia, has been characterized by a more severe course and a failure to respond to conventional treatment. This article describes the case of a patient with essential cryoglobulinemia presenting with acronecrosis with a poor outcome, despite treatment, leading to amputation.

Enfermedad de Kikuchi-Fujimoto: evolución en brotes con respuesta a antipalúdicos / Kikuchi disease: evolution during outbreaks and its response to antimalarial treatment

La enfermedad de Kikuchi-Fujimoto (EKF), también denominada linfadenitis histiocítica necrosante, es un síndrome que se caracteriza por la presencia de adenopatías cervicales, generalmente dolorosas, junto con fiebre y sudoración nocturna. Es una entidad clínica poco frecuente, que afecta principalmente a mujeres jóvenes de procedencia asiática, aunque tiene una distribución universal. Su interés radica, que a pesar de tratarse de un proceso benigno y autolimitado, es necesario de un diagnóstico anatomopatológico de exclusión de otras entidades clínicas como el lupus eritematoso sistémico, tuberculosis y otras como el linfoma o histiocitoma. Se presenta un caso de una EKF, prestando especial atención a su relevancia clínico-patológica y su evolución en brotes, los cuales remiten con antipalúdicos, y en el caso que nos planteamos el uso de hidroxicloroquina en las formas recurrentes (AU)

Kikuchi-Fujimoto disease, also known as histiocytic necrotizing lymphadenitis, is a syndrome characterized by the presence of usually painful cervical lymphadenopathy with fever and night sweats. It is a rare clinical entity that mainly affects young Asian women, although it found worldwide. It is a benign and self-limiting condition; however, its importance lies in its differential diagnosis with other clinical entities such as lymphoma or histiocytoma. We present a case of Kikuchi-Fujimoto disease, with special emphasis on its clinicopathological significance and its evolution during outbreaks. Antimalarial therapy causes remission and we discuss the use of hydroxychloroquine for reoccurring cases (AU)

Enfermedad de Bardet-Biedl asociada a síndrome metabólico y crisis comicial diagnosticada en la edad adulta / Diagnosis of Bardet-Biedl disease associated with metabolic syndrome and epileptic crisis in an adult

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Crioglobulinemia con acronecrosis no asociada a infección por hepatitis C: presentación de un caso / Cryoglobulinemia with acronecrosis not associated with hepatitis C infection: a case report

La crioglobulinemia es una enfermedad rara en la cual se producen inmunoglobulinas monoclonales y/o policlonales que precipitan con el frío. Si bien este fenómeno puede observarse en una gran cantidad de trastornos, se ha asociado más frecuentemente a la infección por el virus de la hepatitis C en más del 90%. El porcentaje restante, llamado crioglobulinemia esencial, se ha caracterizado por curso más severo y falta de respuesta al tratamiento convencional. El presente artículo describe el caso de un paciente con crioglobulinemia esencial que se presenta con acronecrosis, en la que su mala evolución, a pesar del tratamiento, la lleva a la amputación (AU)

Cryoglobulinemia is a rare disease characterized by the production of monoclonal or polyclonal immunoglobulins that precipitate in cold temperature. While this phenomenon can be observed in a large number of disorders, it has been associated with hepatitis C virus infection in more than 90% of cases. The remaining 10%, called essential cryoglobulinemia, has been characterized by a more severe course and a failure to respond to conventional treatment. This article describes the case of a patient with essential cryoglobulinemia presenting with acronecrosis with a poor outcome, despite treatment, leading to amputation (AU)